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The Treatment of Primary IgA Nephropathy: Change, Change, Change

IgA nephropathy (IgAN) is the most common glomerular disease in the world. However, the approach to treatment remains controversial. There has been an explosion of clinical trials over the past decade both to further examine corticosteroid use and usher in additional treatment considerations, including 2 newly approved therapies for IgAN. Sodium glucose cotransporter 2 inhibitors are proving to be effective therapy across proteinuric chronic kidney diseases, and IgAN is not likely to be an exception. Further supportive agents are looking highly promising and so are novel agents that specifically focus on the pathophysiology of this disease, including endothelin blockade, complement inhibition,…

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Articles of Interest

A Kidney Disease’s Genetic Clues Are Uncovered

The key molecular defect in people with IgAN is abnormal O-glycosylation of IgA antibodies. O-glycosylation—in which a sugar molecule attaches to an oxygen atom in the amino acid residue of a protein—plays a role in various physiologic processes. Studies of families have shown that problems in the O-glycosylation of IgA are common in people with IgAN and are largely genetic in origin, although the exact genes involved were unknown.

IgA nephropathy: An interesting autoimmune kidney disease

Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis worldwide. It is a leading cause of chronic kidney disease and progresses to end-stage kidney disease in up to 40% of patients about 20 years after diagnosis. Additionally, IgAN is associated with significant mortality. The diagnosis currently necessitates a kidney biopsy, as no biomarker sufficiently specific and sensitive is available to supplant the procedure.

IgA Nephropathy: Progress Before and Since Berger

Immunoglobulin A nephropathy (IgAN) is linked inextricably to the name Jean Berger, the Parisian pathologist who published the first description of IgAN in 1968. We reflect on the significance of Berger's first report and consider 40 years of progress in our understanding of IgAN since it was published. We also look back to the days before Berger, when IgAN could not have been identified (because there were no techniques for detecting IgA deposits), classification of glomerulonephritis was even more contentious and confusing than it is today, and it is likely that the literature describing focal glomerulonephritis contained many of the cases we would now identify as IgAN.

INSIDE THE N.H.L.; A Devil Battles a Kidney Ailment

The disease is most commonly found in men from their teens to their 30's. Twenty percent to 40 percent of patients develop end-stage kidney failure about 20 years after finding they have it.

Jean Berger – the man who identified IgA Nephropathy (By John Feehally)

Although IgA nephropathy is the name most commonly used for the renal disease we study, many other terms have been used for it over the last 30 years. Most have derived from a description of the features of the disease found on renal biopsy: for example mesangial IgA disease, IgA glomerulonephritis, and IgA-IgG nephropathy. But one title often used in the past for IgA nephropathy stands out: Berger’s Disease, named after Jean Berger, the French pathologist who published the first description of IgA nephropathy as we recognise it today.

Preventing Progression in IgA Nephropathy: A Managed Care Focus on Emerging Therapies

IgAN is the most common glomerular disease and an important cause of kidney failure. Because of the critical interaction between an intrinsic antigen, Gd-IgA1, and circulating antibodies, IgAN is considered an autoimmune disease. Current KDIGO treatment guidelines recommend early detection and initiation of supportive care treatment regimens that optimize the use of antihypertensives that block RAAS in favor of corticosteroid treatments to delay the progression to CKD and ESRD.

Review on epidemiology, disease burden, and treatment patterns of IgA nephropathy in select APAC countries

Immunoglobulin type A nephropathy (IgAN), also known as Berger’s disease, is a kidney disease caused by kidney deposition of immunoglobulin type A (IgA) complexes involving galactose-deficient IgA and resulting in inflammatory tissue damage. IgAN affects the kidneys by attacking the glomeruli and is characterized by persistent urinary abnormalities including microscopic hematuria, gross hematuria, and/or proteinuria.

Ryan's story: living with IgAN

"You get told when you get diagnosed with IgA Nephropathy that most likely nothing will happen. Well, I was one of those people who got it bad. I was completely healthy until I was diagnosed and even during the first year after I was all right. Then they called me in for a blood test in September 2020 and bang, my kidney function had gone from 73 to 30 per cent. It was so quick, and it happened out of nowhere, and I was at 13 per cent by that Christmas.

What is the difference between IgA nephropathy and Henoch-Schönlein purpura nephritis?

Henoch-Schönlein purpura nephritis (HSPN) and IgA nephropathy (IgAN) are considered to be related diseases since both can be encountered consecutively in the same patient, they have been described in twins, and bear identical pathological and biological abnormalities. Apart from the presence of extrarenal clinical signs found only in HSPN, other differences are noticed between the two diseases. The peak age ranges between 15 and 30 years for a diagnosis of IgAN, whereas HSPN is mainly seen in childhood.

Resources

IgA Nephropathy Foundation’

For far too many people, the journey with IgA Nephropathy (IgAN) is a struggle — often filled with misinformation, sometimes years of medical uncertainty, or the feeling of not knowing where to turn. The IgA Nephropathy Foundation was born in 2004 to help fill this void. As the only organization serving patients with IgAN and their care partners, we are steadfast in our commitment to be the go-to source for information, education, support, and hope.

International IgAN Network Research Group

The International IgAN Network Research Group was conceived in 2018 as a group of researchers within the International IgAN Network who support international collaborative research in IgA nephropathy. Our mission is to develop a sustainable model of novel international collaboration between researchers and stakeholders to improve the capacity and innovation in personalized patient-focused research in IgA nephropathy.

Kidney Research UK

gA is normally present in the bloodstream and its main role is to fight infections throughout the body. But in IgAN the body’s immune cells produce abnormally formed IgA. It is not yet known why this happens. Around 30 per cent of IgAN patients will go on to lose kidney function and will require a transplant or life on dialysis. There are currently no reliable tests available to predict which of these patients, known as progressors, will lose their kidney function.

National Institute of Diabetes and Digestive and Kidney Diseases

The cause of IgA nephropathy is unknown, but research suggests that genes and the environment may play a role.2 In some people, the first signs or symptoms of the disease may become noticeable after a cold, sore throat, or other respiratory infection.

StatPearls

mmunoglobulin A (IgA) nephropathy, or IgAN, also known as Berger disease, is one of the leading causes of glomerulonephritis and renal failure. This disease is a prevalent form of glomerulonephritis characterized by the deposition of IgA in the glomerular basement membrane. Immune-mediated damage to the basement membrane results in hematuria, proteinuria, and renal insufficiency. Pathologically, a spectrum of glomerular lesions may be seen, with mesangial proliferation and prominent IgA deposition being the most commonly observed change.